This quality of life, exploratory study describes the experiences and needs of children living with haemophilia in Costa Rica. The World Federation of Haemophilia (WFH, 2012) defined haemophilia as a rare disease, which is an X-linked congenital bleeding disorder caused by a deficiency in the coagulation factor VIII in Haemophilia A and factor IX in Haemophilia B. Diagnosis and management can be difficult as complications occur including musculoskeletal issues, the formation of inhibitors and transfusion-transmitted diseases. Haemophilia impacts the diagnosed child as well as their families. Overprotective families can lead to anxious, dependent children with behavioural issues (Abali et al., 2014). Some children report low self-esteem, depressive symptoms and lower self-perception (Coppola at el., 2011) but overall the majority adapt to their illness successfully (Canclini et al. 2003) and can manage to live almost normal lives with the appropriate treatments (Chandy, 2005).
The Costa Rican health system is public and run by the Ministry of Health (Caja Costarricense del Seguro Social). According to international economic indicators, Costa Rica is classified as a 'developing’ country; but the standards of haemophilia care and treatment are comparable to developed countries according to the World Federation of Haemophilia, as the social security system allows access to treatment for those who may not otherwise be able to afford it. According to Chandy (2005), children with haemophilia in developing countries can have specific characteristics due to limited resources and elevated treatment costs; however, it is possible for a child to live an almost ‘normal’ life with adequate treatment. Children can attend school, participate in sports and cope with mild bleeds. Evaluating Quality of Life (QoL) is a useful way to understand lived experiences and identify the needs of children with haemophilia. QoL scales can be used to determine the effect of treatments that impact on the quality of life for chronically ill populations (Bullinger and Mackesen 2004; Pollak et al., 2006).
Children between six and twelve years of age were selected for this study as this stage involves important advances in moral development, memory, knowledge and thinking processes, and children can provide an accurate description of themselves including comparisons with their social and cultural context and the impact of these in their lives (Papalia, Wendkos and Duskin, 2001). Individual differences and needs become more important and can have an impact on scholarly success (Papalia, et al., 2001). Williams and Chapman (2011) found that children with haemophilia have a heightened sense of being different and can be set apart from their peers and community because of the rarity of their illness. Young people were also aware of differences from their same-sex peers (Williams and Chapman, 2011; Dasi et al., 2016).
Peers and classmates may require education about haemophilia to avoid peer rejection, while children with haemophilia may also benefit from social and self-disclosing skills (Williams and Chapman, 2011). This paper describes the self-reported experiences and needs of children between six and twelve years of age living with haemophilia in Costa Rica and the impact that the illness can have on their lives and their self-concept. This is the first study to be carried out nationwide on children with haemophilia in this country and can inform practice and policy in terms of how best to support children and their families.
This was an exploratory quantitative study. A QoL Scale for children with haemophilia was designed and validated for the Costa Rican context. Items were informed by previous scales used in an international context1. Those items that were relevant to the Costa Rican context and were understandable for a child and their parents were included. An advisory committee including healthcare professionals and members of the Haemophilia Association validated the content of the questionnaires. The questionnaires evaluated children’s sociodemographic data, nuclear family, peer relationships, self-concept, social Likert scale. Open questions were included to identify children’s definition of haemophilia and their selfreported needs.
This study was approved by the Ethics-Scientific Committee at the University of Costa Rica. Potential participants were contacted by phone and the main researcher organised home visits at a date and time to suit families. Families completed consent forms and questionnaires at that visit. Children were given a certificate and gift for participation. Open questions were coded. Quantitative data was input into the social sciences software package SPSS. This paper is focused on the descriptive analysis of questionnaire data.
Due to haemophilia being an X-linked disorder2, all children in this study (N= 32) were boys between the age of six and twelve years. 28 children had haemophilia Type A and four had Type B (Table 1). Children were asked to describe haemophilia. The majority described it as causing bleeds and bruises (31.3%), an illness of the blood (15.6%) and 28.1% said they did not know. Children were asked about their experience of bleeds: 21.9% were extremely bothered by them, 28.1% experienced very severe bleeds, though only 6.3% were confined to bed due to the bleeds.
The majority of children (53.1%) receive treatment by demand (Table 2). In terms of treatment, the majority of children (93.8%) do not know how to inject themselves. Only 6.3% of fathers administered injections to their children compared to 40.6% of mothers. The majority of children receive treatment from a nurse (40.6%) and classified treatment as effective (96.9%).
Children’s perception of hospitals was generally positive (40.6%) though 18.6% were negative. Half of the children expressed upset at having hospital treatment and 81.3% disliked receiving treatment. The majority of children (90.6%) expressed trust in healthcare professionals.
Children were also asked about the level of perceived control over their illness. The majority of children (84.4%) reported having a medium to high level of control. Over half of them (53.1%) think they are informed about their illness, but 28% expressed no perception of control. The majority (62.5%) stated that haemophilia was part of their normal life, 22% did not think haemophilia was normal in their life. Importantly, 75% of children said they were not upset at having haemophilia and only 15% expressed upset.
Children were also asked about their levels of perceived pain, 46% responded as having medium pain while 31% reported the highest level of pain. Some (40.6%) reported highest levels of fear of hurting themselves. The majority of children (56.3%) described being careful with their bodies. The majority (59.4%) felt strong and energetic yet 28% report not being able to participate in sports and games due to their haemophilia.
Regarding social relationships and supports, half of the children expressed the view that they could discuss haemophilia with friends, yet 28% disagreed. Most children had a best friend (65.6%) that worried about them and 43% did not feel different to their peers due to their haemophilia. The majority of children (84.4%) considered their parents overprotective while 65.6% reported a good relationship with them.
Children generally felt proud of themselves (84.4%), content with themselves (56.3%) and considered they had good ideas (56.3%). However, some children (6.3%) disagreed with these statements completely. Children also mentioned perceived needs they had: treatment and availability of treatment (43.8%), scholarly performance (18.8%), participation in sports and extracurricular activities (15.6%), emotional needs (3.1%) and support and affection (3.1%). 15.6% of children reported no additional needs.
The experiences of Costa Rican children with haemophilia are diverse. Most children report positive lives but some children had severe bleeds and a small number of children were confined to bed due to bleed intensity. This suggests that despite the availability of treatments, haemophilia is still a challenge in their lives.
Children defined haemophilia in a concrete way, focusing particularly on visible bleeds and bruises. Children were aware that they lack additional information on their illness. Previous research has identified that younger ill children usually lack knowledge about their own diagnosis (Del Barrio, 1990), as understanding abstract medical concepts like internal bleeds may be impossible without professional help (Williams and Chapman, 2011). It is therefore relevant to include children as information receivers with age-appropriate information and allowing them to express their concerns (Dasi, Ortuno, Cid-Sabatel and Barbero, 2016). Being able to provide accurate description of their illness and the treatment needed may save these children’s lives.
Children between six and twelve years do not self-administer injections and rely on others for treatment. They are still too young to be able to handle and apply treatment correctly; as they develop this skill, it may eventually make a life or death difference in an emergency situation. The majority of children trust medical staff and the treatment they receive. A small number of children are very unhappy at having to attend hospital because of their illness, suggesting that hospital visits can be disruptive and difficult. As previous research has identified, illness and treatment impacts on the quality of life of individuals (Bullinger 2004; Pollak et al., 2006).
Most children described haemophilia as a normal part of their lives, feeling happy and in control. A small group did not feel in control or described haemophilia as a normal part of their lives. About a quarter of children explained that haemophilia makes their life difficult and they may struggle to cope with their illness. This is in line with previous research suggesting that the majority of children adapt to their illness successfully (Canclini et al. 2003; Chandy, 2005) but some children may be in need of additional supports to adjust.
Regarding social supports, the majority of children talk to their friends about their illness and seem to have a ‘best friend’ that cares about them, but a small number did not. The majority of children described positive parental relationships. The majority of children described themselves as feeling strong, proud, content and healthy but a small number of children with haemophilia may struggle with self-perception, experiencing low self-esteem, which is in line with previous findings (Canclini et al., 2003) reported.
Children described some of their perceived needs which included better access to treatment and better outcomes in education and more play. Previous research has suggested that children with haemophilia can have minor deficits in school when they experience more than twelve bleeds per year (Coppola et al., 2011; Abali et al., 2014).
As previous studies have suggested (Chandy, 2005), children with haemophilia can live an almost ‘normal’ life with appropriate treatment. In this study, the majority of children reported good self-perception, emotional well-being and ability to cope with their illness. However, six children were identified as having a greater vulnerability and risk. These children had lower levels in the perception of haemophilia including their own level of knowledge, the control they have over their illness and their perception of haemophilia as normal in their lives. These children also scored lower in social supports and emotional well-being, describing feelings of sadness, being overwhelmed, bad moods, fear of bleeds and insecurity about their haemophilia. The majority of children seem to adapt successfully to their illness but a small number of children may be struggling and reporting a higher level of vulnerability which can have a negative impact on their quality of life. This suggests the need to approach health not as an exclusively biological issues but with biopsychosocial interventions and comprehensive care (Dasi et al., 2016). While this study provides a description of variables that characterise children with haemophilia in Costa Rica, this exploratory study did not determine the correlations or interactions between variables, which may interest future research on the field.
1 WHOQOL-100, World Health Organisation, 1995. KINDL Ravens-Sieberer and Bullinger 1998. KIDSCREEN Ravens-Sieberer 2005. ESCHQoL Schramm et al., 2012. Haemo-Qol Index, Pollak et al., 2006. HEMOFILIA QoL, Remor et al., 2005. HEMOLATIN-QoL, Remor, 2016.
2 X linked recessive inheritance is caused by a mutation in the gene on the X chromosome that causes the phenotype to be expressed only in males as they have only one X chromosome.